Diagnostic search for causes of primary adrenal insufficiency in children

Abstract

Primary adrenal insufficiency rarely occurs in pediatric practice. The clinical diagnosis of the disease is frequently delayed or missed because of nonspecific symptoms early in the course. Hypocorticism should be differentiated from other diseases with similar symptoms or laboratory changes. Hyponatremia is one of the most consistent laboratory signs of adrenal insufficiency in children, while hyperkalemia is detected in half of patients. Detection of low sodium levels in the absence of hyperkalemia often misleads doctors, leading to false diagnosis of «syndrome of inadequate secretion of antidiuretic hormone». Increased thyroid­stimulating hormone level is characteristic of the manifestation of hypocorticism, meanwhile evaluation of the thyroid status is carried out more frequently than the level of adrenal hormones. Therefore, manifestations of Addison’s disease would be suggested to be the symptoms of hypothyroidism. At the same time, intake of levothyroxine in unrecognized hypocorticism is dangerous, as it can trigger an adrenal crisis.In children genetic causes of chronic adrenal insufficiency are the most prevalent. Hypocorticism in children may be the first manifestation of autoimmune and hereditary diseases with multiorgan lesions, so the precise diagnosis may influence the treatment decisions, further examination of the patient and the range of indicators to be monitored. Searching the cause of adrenal insufficiency in children is often a challenge and requires joint work of specialists in different fields. The article presents a review on the diagnostic search for causes of primary adrenal insufficiency in children of different ages.